Cholecystitis, Cholelithiasis and Common Duct Stenosis
in Children and Adolescents
GEORGE W. HOLCOMB, JR., M.D., JAMES A. O'NEILL, JR., M.D., GEORGE W. HOLCOMB, 111, B.A.
A study of 100 patients from 14 months through 18 years of
age with extrahepatic biliary tract conditions who have been
treated from 1950 through 1979 is reported. For discussion,
these have been classified into four groups including acalculous
cholecystitis, nonhemolytic cholelithiasis, hemolytic cholelithi-
asis and stenosis of the common duct. Ninety-nine patients
were operated on and there were no deaths. Except for
unusual contraindications, cholecystectomy is preferred for
acute noncalculous cholecystitis. The largest number having
gallstones were those patients (87%) without hemolytic
disease. Only 13% had an associated hemolytic disorder.
Cholecystectomy is the preferred treatment and common duct
exploration is utilized when indicated. Six children with chronic
relapsing pancreatitis secondary to stenosis of the ampulia of
Vater and two with common duct stenosis are analyzed. Al-
though extrahepatic biliary disorders are usually not con-
sidered in the differential diagnosis of children and adolescents
with vague abdominal pain, it is evident by this large number
of patients that there should be greater emphasis placed on
earlier diagnosis in the future.
HE CHARACTERISTICS OF BILIARY tract disease have
been well documented through the years for
adult patients. During the past 15 years several authors
have called attention to the similarity of symptoms
and physical findings in children and adults with gallblad-
der disease with or without stone formation.12'13'16'24'25'33
However, many surgeons and pediatricians apparently
have not seen nor do they readily consider the possi-
bility of acute cholecystitis, cholelithiasis or stenosis
of the common duct in young patients who present
with vague right upper quadrant pain. Perhaps one
reason for this hesitation is the fact that emphasis was
placed earlier on the belief that hemolytic disease is a
necessary prerequisite of stone formation in children
and adolescents. 13,14,34,39 A review of our experience
reported in 1966 from this institution called attention
to the increasing number of children and teenagers
Presented at the Annual Meeting of the Southern Surgical Associa-
tion, December 3-5, 1979, Hot Springs, Virginia.
Reprint requests: George W. Holcomb, Jr., M.D., Department
of Pediatric Surgery, Vanderbilt University Medical Center,
Nashville, Tennessee 37232.
Submitted for publication: December 7, 1979.
0003-4932/80/0500/0626 $01.00 © J. B. Lippincott Company
626
From the Department of Pediatric Surgery,
Vanderbilt University Medical Center,
Nashville, Tennessee
being diagnosed as having extrahepatic biliary con-
ditions who presented with unexplained abdominal
symptoms.24 Since that time collective reviews from
institutions in this country and Europe have empha-
sized the increasing discovery of gallbladder and
common duct diseases in children.
Clinical Material
A retrospective study of 100 patients 18 years of age
and under with extrahepatic biliary tract conditions
has been conducted at Vanderbilt Medical Center and
its affiliated hospitals. This group includes patients with
acalculous cholecystitis, cholelithiasis and common
duct obstruction secondary to stones or stenosis with or
without pancreatitis. Biliary atresia and choledochal
cyst have not been included. This study incorporates
44 patients seen from 1950 through 1965 and reported
in 196624 with the addition of 56 new patients treated
since that time. One hundred patients between the ages
of five weeks and 18 years, all but one of whom were
operated on for extrahepatic biliary tract disease, have
been evaluated. These patients are divided into four
major groups as seen in Table 1.
Acalculous Cholecystitis
Seven patients aged 14 months through 15 years were
found to have marked inflammatory disease of the gall-
bladder without stones. There was a male to female
patient ratio of 5:2. Their symptoms and operative
findings are listed in Table 2. A mistaken diagnosis of
acute appendicitis was made in three boys preopera-
tively whose symptoms were less than 48 hours' dura-
tion. Both the 6- and the 10-year-old girls complained
of intermittent pain in the right upper quadrant and
Vol. 191 . No. 5 BILIARY TRACT DISEASE IN CHILDREN 627
TABLE 1. Extrahepatic Biliary Tract Disease in 100 to contribute to the obstruction in two patients sug-
Children and Adolescents gesting a possible congenital anomaly. Considerable
Number Per Cent edema with bile staining was observed in the retro-
Group Patients of Total peritoneal tissues surrounding the common duct and
I. Noncalculous cholecystitis 7 7 head of the pancreas in each patient. Bile culture re-
II. Cholelithiasis (nonhemolytic) 74 74 sulted in heavy growth of Escherichia coli in the
III. Cholelithiasis (hemolytic) 11 11 14-month-old infant who was septic with severe
IV. Stenosis diarrhea (Table 2).
common hepatic duct 2 2
common duct, ampulla 6 6 Cholecystectomy was performed in each patient.
There were no immediate postoperative complications
and all have remained symptom free except for one
cholecystogram identified a nonfunctioning gall- 15-year-old boy who three years following the chole-
bladder. Jaundice was not noted in any of these patients cystectomy developed pancreatic calculi and required
although this has been reported by others to be an a distal pancreatic resection (Peustow). He, like his
accompanying feature of this disease.29'32'33'40'4' Fever, father, was found to have hereditary pancreatitis.
nausea, vomiting, diarrhea, dehydration and marked Acalculous cholecystitis has been described by
subhepatic tenderness were the most prominent others in association with many different conditions,
findings. including sepsis, burns,26 trauma,3233 and following
At operation the gallbladder was inflamed, markedly other operative procedures." .32 Obstruction of the
distended in each, and did not collapse with manual cystic duct with gallbladder distention from enlarged
compression. Gangrenous changes without perforation lymph nodes, congenital narrowing or tortuosity have
were observed in one 14-month, one 11-year and one been mentioned as contributing causes of chole-
15-year-old boy. A small tortuous cystic duct was found cystitis.'3'24'40 The two factors which seem to be most

TABLE 2. Acalculous Cholecystitis in Children

Age
(yr.) Follow-up
Patient and Sex Symptoms Preoperative Findings Operative Findings Study
1. L.H. 1.3, M Tender mass, RUQ, Temp. 103, WBC 18,100 per cu mm Distended inflamed gallbladder, No symptoms
dehydration, diarrhea cholangiogram, normal duct,
culture bile: heavy E. coli.
2. P.P. 5, M Pain in right upper quadrant WBC 20,000 per cu mm Distended, inflamed gallbladder, No symptoms
for 24 hr with muscle guarding. with 82% segmented cystic duct very tortuous, and
Suspected appendicitis. neutrophils. cholecystogram showed cystic
duct obstruction before
mobilization.
3. R.P. 6, F Two weeks' pain RUQ, nausea, Nonfunctioning gall- Distended, inflamed gallbladder. No symptoms
vomiting. bladder. WBC 9,400
per cu mm.
4. J.S. 10, F Intermittent colicky pain in right Nonfunctioning gall- Dilated, inflamed gallbladder with No symptoms
upper quadrant. Appendectomy bladder on two small cystic duct. Mucosa
one year before for right occasions. normal.
abdominal pain with normal
appendix.
5. P.A. 11, M Right-sided abdominal tenderness WBC 17,000 per cu mm Gangrenous, edematous No symptoms
for 12 hr., nausea and vomiting. gallbladder, normal appendix.
Suspected appendicitis
6. T.D. 11, M Pain and tenderness in right WBC 10,500 per cu mm; Edematous gallbladder with No symptoms
upper quadrant for 48 hr. total bilirubin 0.55 ulceration of mucosa. Acute
Suspected appendicitis. mg/dl cholecystitis.
7. D.G. 15, M Severe upper abdominal pain with WBC 14,500; amylase- Gangrenous cholecystitis. Three years later
nausea. normal. Father has required distal
familial pancreatitis pancreatectomy
with stones. for pancreatic
stones
(Peustow).
 HOLCOMB, O'NEILL AND HOLCOMB Ann. Surg. * May 1980
628
24 children who are seriously ill. In those patients with
23 jaundice, common duct exploration is not indicated
22 unless the duct is dilated. Cholangiography by injection
21
20 of the dilated gallbladder or with cannulation of the
19 cystic duct may be of value in such situations. Jaundice
18 and symptoms usually subside promptly following
17 ; E MALE
6 removal of the gallbladder.
* FEMALE
15
NUMBER 14
OF
Cholelithiasis
13
PATIENTS 12 Seventy-four young patieuits in this study without
ll
10 hemolytic disease were proven to have cholelithiasis.
9 Ages ranged from five through 18 years (Fig. 1). The
8 sex ratio is overwhelmingly female vith 69 girls to five
7
6 boys. Racial distribution is predominantly Caucasian
5 with only I2 blacks. A family history was positive in
4 only five instances. An additional 11 children were
3 found to have gallstones associated with hemolytic
2
I disease or a total of 85 children and adolescents with
m w all types of cholelithiasis.
4 5 6 7 8 9 10 11 12 13 14 15 116 17 1
AGE AT TIME OF OPERATION
(YEARS) Nonhemolytic Cholelithiasis
FIG. 1. Age and sex of 54 children and adolescents with non- Of 74 patients with idiopathic gallstones, the pre-
hemolytic cholelithiosis. dominant symptom in 38 was epigastric and/or right
upper quadrant pain. This pain radiated to the sub-
important in producing cholecystitis are partial or scapular region in 23 additional patients and the iight
complete o"'struction of the cystiL duct and subsequent lower quadrant simulating appendi itis in six children.
development of acute inflammation. Other contribu- The remaining seven had no symptoms. Nausea and
tory factors include dehydration, bile statis and an vomiting occurred in 22 and intolerance to fatty foods
accompanying septic disease. was experienced by 16 patients. Twenty-fil girls were
While only one of our patients had an associated overweight. A total of 38 patients experienced symp-
disease, a 14-month-old child with severe diarrhea, toms for three months or less and 18 for six months
sepsis and dehydration, Ternberg and Keating dis- before the diagnosis of choielithiasis was made. It is
covered a history of illness preceding cholecystitis disturbing that gallbladder disease existed for one to
in 45/74 patients (60%).40 It is of interest that jaundice four years in 17 (23%) patients, one of whom was
was present in 30% who had a preceding illness but mistakenly told she should see a psychiatrist because
in only one patient without a separate primary disease. she did not have an organic disease.
Unfoitunately, -_ute cholecystitis is often over- It has been claimed that it is difficult to find docu-
shadowed by associated diseases and the correct pre- mented evidence of cholelithiasis in children in the
operative diagnosis is frequently overlooked.19 absence of a hemolytic procesi. In the present study,
Differentiation from hepatitis, choledochal cyst, however, we have found 14/74 children between five
peritonitis or an acutely inflamed appendix located in and 15 years of age with gallstones and no evidence of
the subhepatic area is difficult but usually can be done hemolysis. The sym; toms and findings of these 14
if the clinician maintains a high index of suspicion for young children are detailed in Table 3. It is of interest
inflammation of the gallbladder. Diagnostic studies that the 5-year-old boy who co tiplained of intermittent
including cholecystogram and abdominal ultrasound episodes of pain in his right subcostal area for four
will bOe of assistance. months very likely would not have been diagnosed
Since perforation of the zillbladder from gangrenous because of his young age if radiopaque stones had not
changes is always possible,13'29'40 we have concluded been noted on the abdominal films. The remaining 60/74
that this condition should be managed by abdominal patients in this gr ,p were teenagers between 16 and
exploration rather than by nonoperative treatment. Al- 18 years of age.
though cholecystostomy has been reported with some Pregnancy was recorded in 45 (65%) of the 14-18-
success,- we prefer cholecystectomy. This procedure year-old girls with cholelithiasis. None was found to
can be accomplished safely and without delay even in have hemolytic disease. Thirteen teenagers initially
Vol. 191 * No. 5 BILIARY TRACT DISEASE IN CHILDREN 629
TABLE 3. Details of 14 Children 15 Years and Younger wr'ith Nonhemolytic Gallstones
Age
(yrs.) Symptoms
and
Patient Sex Findings Operation Pathology Convalescence

1. C.F. 5, M 4 mo. colic, sickle prep negative Cholecystectomy, Cholelithiasis Hydrops GB Uneventful
cholangiogram 2 stones, "milk calcium"
Radiopaque G. stones Appendectomy
2. L.G. 9, F Intermittent jaundice, pain Transduodenal Dilated common duct Developed common
since age 3 sphincteroplasty and duct stones 9 yrs.
removal stone ampulla later
Bil. 6.5 mg/dl, Amylase 757
Somogyi
3. N.L. 11, F Pain-subcostal, radiate to back, Transduodenal Dilated common duct Uneventful
jaundice 10 days sphincteroplasty.
Removal stone
ampulla
4. D.P. 11, F Subcostal pain 18 mo. Intolerance Cholecystectomy. Dila- Dilated common duct and Uneventful
fat foods tation sphincter Oddi, stone ampulla
appendectomy Cholelithiasis
5. J.U. 11, F Subcostal pain 10 mo. Cholecystectomy, Cholecystitis 3 stones, Uneventful
Cholecystogram nonfunctioning; appendectomy largest 5 mm
ultrasound- stones
6. R.H. 12, F Subcostal pain 2 yrs. Cholecystectomy, Cholecystitis, multiple small Uneventful
Some N&V appendectomy stones

Radiolucent stones, O.C.G., 25 lbs.

overweight.
7. S.B. 13, F ColicPain,N&V, Diarrhea lOdays, Cholecystectomy, Cholecystitis, common duct Uneventful
Pancreatitis. Amylase 211 cholangiogram, normal, 2 large 1.5 cm.
(K.A.), radiolucent stones, appendectomy stones and numerous
O.C.G. Obese small
8. W.G. 13, M Subcostal pain 4 mo. Radiolucent Cholecystectomy, Cholecystitis, many small Uneventful
stones O.C.G exploration CD-No stones
stones
9. J.T. 14, F Dull epigastric pain-3 mo. Fat Cholecystectomy, Cholecystitis, 40 1-2 mm Uneventful
Intolerance appendectomv stones

Radiolucent stones, O.C.G.

10. C.C. 14, F Subcostal back pain 5 mo. Started Cholecystectomy, Cholecystitis, multiple small Uneventful
just after baby born. Fat cholangiogram stones
intolerance
I 1. J.C. 15, F Epigastric & back pain last Cholecystectomy, Cholecystitis, multiple Uneventful
trimester. Delivered baby 7 wks. appendectomy stones, 2-4 mm
PTA. Radiolucent stones, O.C.G
12. C.V. 15, F Subcostal pain intermittentfor yr. Discharged to return for
Radiolucent stones O.C.G cholecystectomy
13. L.B. 15, F Subcostal pain 1 yr Cholecystectomy, Acute cholecystitis, many Uneventful
appendectomy small stones
Opaque stones, WBC 19,850
14. C.F. 15, M Subcostal Pain 3 mo Cholecystectomy, Common duct normal Uneventful
appendectomy, hydrops gall bladder stone
Nonvisualization with O.C.G., cholangiogram impacted cystic duct
Ultrasound stones

developed symptoms during pregnancy, 23 within six (65%) of girls with gallstones associated with pregnancy
months of delivery and nine experienced symptoms in the present group is similar to that reported in 1966.24
more than six months postpartum. This high percentage The leukocyte count in this group of 74 patients was
 HOLCOMB, O'NEILL AND HOLCOMB
630
elevated above 10,000/mm3 in only 12 children. In 11
patients the total serum bilirubin level was greater than
1.2 mg/dl; the remainder had normal values. The serum
cholesterol was elevated in only two 16-year-old girls
being 287 mg/dl and 320 mg/dl respectively.
Radiographic evaluation identified radiopaque cal-
culi in 20 patients and radiolucent stones in 35. The dye
failed to concentrate in nine patients. Intravenous
cholangiogram or ultrasonic studies were used to con-
firm stones in this group. The remaining patients had
outside roentgenograms made and were not available
for review.
Cholecystectomy was performed in 73 nonhemolytic
patients. One 15-year-old girl was discharged after
cholecystogram revealed stones and she will return
later for operation. Incidental appendectomy was per-
formed 21 times. Operative cholangiogram was ob-
tained in 21 patients and this study was helpful in
identifying stones in the common duct or areas of
stenosis. Common duct exploration using the accepted
criteria for adults was thought necessary in 16 of these
patients. A stone was found in the distal common
duct in five girls, ages nine years (1 patient), 11 years
(2 patients) (Table 3) and 18 years (2 patients). Trans-
duodenal sphincteroplasty or dilatation of the sphincter
was required to remove the impacted stone in each
instance. In this group of 73 patients operated on for
cholelithiasis, there were no deaths. Two wound in-
fections occurred. One girl developed intestinal ob-
struction from adhesions and required another opera-
tion for lysis three years later. Only one 9-year-old
girl developed a common duct stone postoperatively
(Table 3). Follow-up studies revealed that all patients
were able to eat a regular diet without discomfort or
dyspepsia.
Histologic studies revealed acute inflammation in
only three gallbladders. Chronic cholecystitis with
scarring and thickening was observed in 56 gall-
bladders. A normal mucosa was found in two instances
in spite of the presence of stones. Cholesterolosis was
evident in three patients. Hydrops of the gallbladder
with a stone impacted in the cystic duct was found on
four occasions. "Milk of calcium" was found in two
girls, 11 and 18 years of age. The gallstones were com-
posed of cholesterol primarily and varied in color from
pale yellow to dark yellowish brown with a few having
a greenish tint. The calculi were predominantly 1-5
mm in diameter in 45 patients, 0.5-3.0 cm in 19, al-
though on occasions a larger stone was mixed with some
of the smaller ones. The size was not recorded in the
remainder.
Six teenagers in this group were originally admitted
with acute pancreatitis. Following investigative studies,
they were found to have cholelithiasis. All six girls
were from 13 through 18 years of age. Three were post-
Ann. Surg. * May 1980
partum and three were obese. None were clinically
jaundiced. The acute pancreatitis was allowed to sub-
side and cholecystectomy was performed later in each
patient. No stones were found in the common duct of
any of these girls, although operative cholangiograms
were obtained in four and the duct was explored in two.
Pancreatitis did not recur and convalescence was un-
eventful.
Discussion
Although cholelithiasis is usually considered to be a
condition that occurs in adults, it has been known to
exist in the pediatric age group for over 240 years. Per-
haps the first description of gallstones in a child in the
English literature was recorded by Gibson in 1737.10
This Edinburgh surgeon reported the case of a 13-year-
old boy who was examined by him 13 months following
trauma to the right side of his chest. Gibson described
removing "three Scotch pints of water of a greenish
hue" by paracentesis from the child's abdomen. Two
days later the patient died and autopsy revealed a dis-
tended common duct filled with many spongy, yellow
stones. Little interest in this subject was evident until
Potter's report appeared in 1938.30 Ulin et al. com-
pleted a comprehensive study of cholecystitis in children
in 1952 and accepted 326 cases reported in the literature
as having a valid diagnosis.4' Most individual re-
views, however, have contained reports of fewer than
30 patients. One of the youngest patients, a 6-week-old
infant with a perforated gallbladder and stones in the
common duct, was reported by Snyder et al.38 This
baby had a normal osmotic fragility test.
Early in the twentieth century, typhoid fever was
thought to be a common cause of cholecystitis but
this occurrence is so infrequent today that it is not a
significant factor. Various congenital anomalies of the
cystic duct have also been implicated in gallstone
formation. Glenn and Hill suggested that dehydration
and biliary stasis with bile concentration promote stone
formation." Small is of the opinion that the liver
secretes bile supersaturated with cholesterol which is
precipitated in the gallbladder where this substance
contributes to the production and continued growth
of cholesterol stones.36 In a study of 27 children under
16 years of age with nonhemolytic cholelithiasis,
Hagberg et al. discovered low serum lipid values in
each of these children.15 Also, these authors deter-
mined that hypercholesterolemia did not seem to be an
etiologic factor causing gallstones in childhood.
Several reports have suggested a relationship be-
tween gallbladder disease and obesity. The association
of obesity with nonhemolytic cholelithiasis was found
in 33% of our patients.
Peterson, as early as 1910, called attention to the
frequent association of pregnancy to gallbladder
Vol. 191 No. 5 BILIARY TRACT DISEASE IN CHILDREN
disease in adults.27 Other reports in recent years have
emphasized the high incidence of gallstones developing
in parous teenagers.12'24'33 Indeed, pregnancy has been
determined to be cholestatic.35 Gallstones have been
produced in rabbits experimentally by the prolonged
injection of progesterone and estradiol simulating the
hormones manufactured by the placenta during preg-
nancy.20 Whether the placental hormones decrease
cholesterol solubility or relax smooth muscles of the
common duct allowing regurgitation of pancreatic and
duodenal secretions is not known. Whatever the
physiologic mechanism, pregnancy and obesity seem to
accentuate any metabolic defect or hereditary pre-
disposition for gallstone formation.
While it is doubtful that the exact mechanism of
gallstone formation can be identified as a single factor,
at present it must be thought of as a combination of
interacting processes including dehydration, endo-
crine, transient hepatic dysfuntion, hereditary, dietary,
inflammatory and sex related influences which affect
the composition of bile.
Oral cholecystography is the preferred diagnostic
test and should be used more often in combination
with gastointestinal series in young children and
teenagers who complain of epigastric or subcostal dis-
comfort and dyspepsia. This would reduce the delay
from the onset of symptoms to operation which ranged
in this group from one to four years in 23% (17) of the
patients. Although nonvisualization of the gallbladder
is strong, presumptive evidence of disease, it is not
definitely diagnostic. However, additional investi-
gative procedures such as abdominal sonography and
intravenous cholangiography should determine the
presence or absence of gallstones with reasonable
accuracy.
Cholecystectomy is advised for young patients re-
gardless of the cause or the age at onset of symptoms
except for those with congenital heart disease or other
serious illnesses who are a greater risk. We have no
enthusiasm for leaving a gallbladder intact when stones
are present, even though they may be small enough
to pass down the common duct.2 Operative cholangio-
grams are often of great value to the surgeon and
should be obtained if there are small gallstones, history
of jaundice or dilatation of the common duct. If the
common duct is dilated, contains a palpable stone or
if one is suspected from the operative cholangiogram,
the surgeon should not hesitate to explore the duct
in children or teenagers provided this is done with
extreme caution.
Cholelithiasis and Hemolytic Disease
In addition to the 74 patients mentioned above, there
were nine girls and one boy with spherocytosis and one
girl with sickle cell disease. Ages of the children with
631
spherocytosis ranged between six and 15 years with an
average of 9.8 years. The girl with sickle cell disease
was 17 years old. Four of the 10 were jaundiced at
operation. Gallstones were calcified and seen on plain
roentgenograms in four children. Cholecystogram
identified radiolucent stones in four others. Stones
were palpated by the surgeon during splenectomy in
two children which prompted him to proceed with
cholecystectomy. One girl at age five years had a
splenectomy for spherocytosis but no stones were
palpated in the gallbladder during the operation. Ten
years later she was referred to a urologist because of
back pain and was found to have a normal renal col-
lecting system but radiopaque gallstones were ob-
served. Cholecystectomy was performed without
incident. Because abdominal pain associated with
sickle cell crisis may resemble acute cholecystitis,
it is advisable to obtain a cholecystogram or ultrasonic
study in any patient with known sickle cell disease
who experiences abdominal discomfort.1 Similar
studies should be performed for children over four
years of age who undergo splenectomy for spherocytosis.
Various anemias such as spherocytosis, sickle cell
anemia and thalassemia are well recognized conditions
associated with gallstone formation. However, chil-
dren with these anemias constituted only 13% of the
patients with cholelithiasis in this series. This finding
is not in agreement with others who have maintained
that the most common cause of cholelithiasis in child-
hood is a hemolytic process. 13,14,25,28,34,39 When splenec-
tomy is performed the gallbladder should be palpated
routinely and if stones are present simultaneous
cholecystectomy is indicated. The combined operation
of splenectomy and ch-olecystectomy was not associ-
ated with mortality or significant morbidity in this
group of ten children. An upper midline or long, left
subcostal incision proved satisfactory in performing
both procedures. With the anesthesia expertise cur-
rently available and proper supportive measures,
simultaneous removal of the spleen and gallbladder
is indicated rather than staging these operations.
Stenosis of Common Duct
Stenosis of Ampulla of Vater
Over the last eight years we have encountered six
children with ampullary obstruction associated with
chronic relapsing pancreatitis where the presenting
finding in all but one child was jaundice (Fig. 2). Due
to the absence of severe pain, pancreatitis was not
suspected initially. These six cases have been analyzed
in order to determine the ideal diagnostic and thera-
peutic approach to this condition (Table 4).
Clinical Material . Five boys between the ages of four
months and eight years and one 9-year-old girl have
 Ann. Surg. May 1980
632 HOLCOMB, O'NEILL AND HOLCOMB *

atology, the mistaken diagnosis of chronic hepatitis

was entertained often. Only one patient had any degree
of hyperamylasemia while one other patient had a very
STENOSIS HEPATIC mild, transient elevation in the serum amylase. The
other four children had no elevation in serum amylase
nor were amylase clearances elevated. Serum lipid
5 y M profiles were performed in each patient and only one
8y F was found to have an abnormality, a Type I hyper-
lipoproteinemia.4 Bile cultures were negative in all
six patients.
Upper gastrointestinal examinations were performed
in each child but in only one instance was pan-
creatitis suspected because of the distortion of the
STENOSIS AMPULA duodenal curve. Intravenous cholangiogram in one
4mo M patient revealed dilated intrahepatic ducts but no
2 y M
extrahepatic duct visualization was evident. Ab-
3y M
dominal ultrasonography indicated pancreatitis in one
4y M
patient, although this study was used in a total of
three children.
8y M
The preoperative diagnosis of chronic fibrosing pan-
9y F
creatitis with biliary obstruction was made in only three
FIG. 2. Schematic drawing indicating location, ages and sex of of the six patients. However, the diagnosis was con-
children with stenosis of the common duct. firmed at operation in all patients. Operative cholangi-
ography usually performed through the dilated gallblad-
been evaluated. Four of the six patients had only mild der revealed severe narrowing of the intrapancreatic
abdominal pain either with or without nausea and portion of the common duct in five and complete
vomiting, and in only one instance was the pain con- obstruction in the sixth child. In two instances
sidered to be clinically significant. Two patients had the papcreatic ducts were visualized but only once did
no abdominal complaints whatsoever. Five of the six the ducts appear to be abnormally dilated. Because
children had clinical jaundice and all had elevation in the intrapancreatic portion of the common bile duct
serum bilirubin ranging from 1.0 to 14.7 mg/ml. The was narrowed, it was difficult to evaluate the status
pattern was one of conjugated hyperbilirubinemia with of the sphincter of Oddi with accuracy in these young
elevation in alkaline phosphatase indicative of ob- children. With this limitation in mind, the sphincter
structed jaundice. However, the jaundice was charac- did appear to be stenotic in all. Characteristically,
teristically intermittent and with such mild symptom- the common bile duct and comnmon hepatic ducts were

TABLE 4. Details of Six Children with Stenosis Ampulla of Vater and Idiopathic Chronic Fibrosing Pancreatitis
Age Peak
at Bili- Amylase
Patient Onset Symptoms Signs rubin Elevated Operation Follow-up
1. 9 yrs. Mild abdominal Jaundice, acholic stool 4.5 No Sphincteroplasty Well 21/2 years postop.
pain, nausea
2. 4 mos. None Jaundice, acholic stool 2.4 No Sphincteroplasty Well 7 years postop.
3. 8 yrs. Abdominal pain, Fever, jaundice, acholic 3.9 No Sphincteroplasty Well 1 year postop.
vomniting stool
4. 4 yrs. Mild abdominal Jaundice, light stool 6.6 No Sphincteroplasty Well 2 yrs. postop.
pain
5. 2 yrs. Severe abdominal None 1.0 Yes Sphincteroplasty Mild pancreatitis twice
pain up to 18 mos. postop.,
OK last 4 yrs.
6. 31/2 yrs. None Jaundice, acholic stool 14.7 Yes Choledochoduodenostomy Too recent
Cholecystectomy
Sphincteroplasty
Vol. 191.o No. S BILIARY TRACT DISEASE IN CHILDREN
thickened and fibrotic with the appearance of chronic
inflammation. The ducts appeared to be of normal
calibre in one instance, markedly narrowed in the
second and dilated at least three times normal size
in the remaining four patients. Routinely, the common
bile duct was explored and transtduodenal sphincter-
oplasty performed. In the single patient who had com-
plete biliary obstruction, cholecystectomy and chole-
dochoduodenostomy were performed in addition to
sphincteroplasty.
All of these patients recovered rapidly without major
complications. In one instance, serum amylase re-
mained elevated for 12 days before returning to normal
but none of the others had postoperative pancreatitis.
Biliary obstruction resolved within 10 days in all
instances. Five patients had sufficiently long follow-up
ranging from one to seven years to be meaningful. One
child had two episodes of mild pancreatitis up to 18
months postoperative but has remained well for the
succeeding four years. While low fat diets were utilized
initially, all patients have returned to normal diets and
are free of any symptoms. None of the patients have
demonstrated endocrine or exocrine dysfunction.
Discussion Stenosis Ampulla. Although chronic
fibrosing or relapsing pancreatitis has been considered
to be rare in childhood, we have had the opportunity
to see six cases in the last eight years. None of these
were familial or hereditary.22'3' The presence of jaun-
dice and the lack of symptoms misdirected the clinician
toward hepatitis in the majority of cases. Radiographic
or nuclear medical studies have proved to be of limited
value in our hands but probably should be obtained
in evaluating these jaundiced children. Diagnostic
laboratory tests were helpful only in determining the
presence of obstructive jaundice. It appears however
that the most helpful diagnostic and therapeutic
procedure in children with intermittent conjugated
hyperbilirubinemia of unexplained nature is explora-
tory laparotomy and operative cholangiography. The
pancreas in these patients has routinely been firm,
nodular and markedly thickened with gross appearance
of chronic and subacute inflammation.
The exact cause of this disorder is not known but
some type of ampullary stenosis which may lead to
reflux of bile into the pancreatic ducts is at present
the most appealing explanation. For this reason, we
have depended primarily on sphincteroplasty as the
operative procedure of choice. It is known that stenosis
of the ampulla of Vater may cause pancreatitis and
Donahoe and Hendren have reported perforation of the
common duct in a 2-month-old infant with this con-
dition.8 Carnevali a,nd his group have shown that
ductal decompression may relieve the sequence of
events leading to this disease.5 However, in the
633
absence of knowing specific ductal pressures, one is
necessarily in the position of speculating and using
clinical observations to guide the course of treatment.
In a previous report, Hendren suggested that ob-
struction was a contributory factor in childhood
pancreatitis.'8 Contrary to this view, however, is the
uncertainty regarding obstruction being the primary
factor since cases have been reported in which there
was no demonstrable obstructive mechanism as with
the case reported by Williams et al.43 The studies of
Wainwright suggest that chronic fibrosing pancr,atitis
is due to intermittent episodes of focal acinar rppture
with subsequent scarring and ductal narrowing.42
Certainly it is possible that bile reflux due to a common
channel and perhaps sphincteric stenosis might be
responsible for this condition. As far as we can deter-
mine, other possible causes have been excluded. Hy-
perlipemia as seen with hereditary pancreatitis was
only vaguely suggested in one of our six patients. Amino-
aciduria was ruled out. Hyperparathyroidism, trauma,
infectious and drug-related causes were eliminated.
None of the patients had mucoviscidosis.
The ideal surgical approach is uncertain and only
time will tell what is most appropriate. Our approach
has been based on the clinical observation that ob-
struction was the primary finding. Sphincteroplasty
has been associated with long-lasting relief in our
patients and those described by Robichek3' and
Bartlett.3 While other forms of caudal pancreatitic
drainage may also have merit, it remains to be seen
whether this procedure will be required in this group
of patients.
Stenosis of Common Hepatic Duct
Two additional patients who presented with inter-
mittent jaundice of 12 months' duration were found to
have stenosis of the common hepatic at its junction with
the cystic duct (Fig. 2). One was a 5-year-old boy and
the other was an 8-year-old girl. Each child was symp-
tom free but was jaundiced and thought to have
chronic, active hepatitis. Laboratory findings of con-
jugated hyperbilirubinemia, an elevated alkaline phos-
phatase and cholesterol with a normal serum amylase
indicated an obstructive pattern. Abdominal explora-
tion revealed what appeared to be a congenital stenosis
at the junction of the common hepatic and common bile
duct. As far as could be determined, there was no
element of pancreatitis. The 5-year-old boy had four
separate operations directed toward excision of the
diaphragm-like stenosis with maintenance of bile flow
through a T-tube, each of which resulted in stricture.
Finally, when he was 17 years of age a Roux-en-Y
choledochojejunostomy was performed with an un-
634 HOLCOMB, O'NEILL AND HOLCOMB
eventful recovery. The 8-year-old girl had a Roux-en-Y
choledochojejunostomy as well as cholecystectomy.
Her response was immediately favorable. She has been
followed closely for the last eight years and con-
tinues to do well.
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DR. MARK M. RAVITCH (Pittsburgh, Pennsylvania): I would like
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the adults with acalculous cholecystitis, in whom there usually has
been preceding trauma, either on the battlefield or in the operating
room with a nonbiliary tract operation.
Dr. Holcomb pointed out that gangrene occurs in some of these
children, and that it probably is unwise to treat these expectantly,
or even with simple cholecystostomy. I wonder, in fact, how many of
these he cultured, and whether he obtained any organisms. Some-
Ann. Surg. * May 1980
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times, indeed, this follows shortly after streptococcal infection of no
great clinical consequence.
If I understood his figures correctly with respect to common duct
stones, he had the astonishing incidence of 6.8%. Now, did all these
patients have cholangiography, or is it a fact that the incidence
was very much higher than that? If they did, we'd expect another
7 or 12% over all, which would be extraordinarily high.
DR. J. ALEX HALLER, JR. (Baltimore, Maryland): I would like to
focus on the fact that in this particular series there was a very
small number of patients who had sickle cell disease. This, I think,
represents a somewhat different series from many which have been